889 BRADYARRHYTHMIA IN MITRAL VALVE PROLAPSE TREATED WITH A PACEMAKER MAXWELL L. GELFAND, M.D. AND HOWARD KLOTH, M.D. New York Infirmary and New York University Medical Center New York, New York M SITRAL valve prolapse receives increasing attention. This entity has many names, including systolic click-late systolic murmur syn- drome, the floppy valve syndrome, and the billowing mitral leaflet syn- drome.3'4'5'7'12'19 It is an important cause of incapacitating chest pain with refractory arrhythmias. Two patients with mitral valve prolapse presented with the manifesta- tions of a sick sinus syndrome, with recurrent severe alternating brady and tachyarrhythmias. Both were treated with propranolol which increased the bradycardia and aggravated one patient's syncopal attacks. To overcome the latter, which may have resulted in a life-threatening seizure, a perma- nent transvenous pacemaker was implanted in both patients. Thereafter, propranolol was continued without ill effects and improvement of symp- toms. CASE 1 R. G., a 61-year-old white woman, was admitted to the University Hospital in 1976 because of chest pain, weakness, and syncope present for about two years. She was previously at another hospital where a diagnosis of mitral systolic click syndrome was made. A Holter electrocardiogram demonstrated a bradycardia of 48 to the minute, with one episode of ventricular coupling (Figure 1). She also demonstrated episodes of atrial tachycardia with recurrent sinus bradycardia and sinus arrest, and had several syncopal attacks. Her past history revealed a thyroidectomy for thyroid carcinoma in 1964, and a mastectomy in 1970. She was receiving 10 mg, of propranolol four times a day for her symptoms upon discharge from the hospital and continued without benefit. Physical examination on admission demonstrated a thin, anxious woman complaining of chest pain and palpitations most of the day. The pain was Vol. 54, No. 9, October 1978 890 M.L. GELFAND AND H. KLOTH t~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~ +1lXl ~ ~-i :1 **1* --1 t ILJIAt--tn- -v _!_ ---L--LJ-±Li5Il-'- K frt STOCK 9500 Fig. 1: Bradycardia with one episode of ventricular coupling not typical of angina pectoris and neither radiated nor was related to effort or eating. It was dull in character, continuous, and not affected by ni- troglycerin. Eyes, ears, nose, and throat were normal. The neck revealed an old thyroidectomy scar. The chest disclosed evidence of a left mastec- tomy and pes excavatum. The heart was not enlarged to percussion. The rhythm was of sinus origin and the rate was 43 to the minute. A systolic murmur was heard at the apex but no click could be detected. The blood pressure was 120/80 mm. Hg. The lungs were clear to percussion and auscultation. The abdomen revealed no masses and the extremities were free from edema or inflammation. The reflexes were normal. A chest roentgenogram was within normal limits except for pes ex- cavatum. An electrocardiogram showed bradycardia with a rate of 45, but otherwise was normal (Figure 2). Routine laboratory data were unremark- able. An echocardiogram demonstrated a prolapse of the posterior leaflet of the mitral valve (Figure 3). On May 5, 1976 a 24-hour Holter tape electrocardiogram documented a bradycardia with occasional ectopic ven- tricular contractions and corresponding symptoms of palpitation and chest pain (Figure 4). The heart rate was 40 beats to the minute. Because the patient had bradycardia and persistent annoying symptoms with small doses of propranolol, a permanent transvenous pacemaker was inserted on May 11, 1976. Larger doses of propranolol then successfully controlled the tachyarrhythmias and chest pain without symptomatic bradyarrhythmia. Bull. N.Y. Acad. Med. BRADYARRHYTHMIA 891 aVL aV F Fig. 2: Bradycardia with a rate of 45 CASE 2 D. M., a 53-year-old white woman, was admitted to the New York Infirmary in 1977 complaining of searing pain in the chest associated with severe palpitations. These symptoms recurred almost daily and were not related to effort, exertion, or food intake. Similar episodes had occurred about two years before, and had recently recurred with an increase in frequency and duration but without dyspnea or syncope. An electrocar- diogram on several occasions revealed atrial flutter with a 2:1 and 3:1 conduction interspersed with periods of normal sinus rhythm, first degree atrioventricular block, and occasional long sinus pauses. For this she was treated with quinidine with very little success. Past history revealed diag- nostic uterine curettage in 1969 and excision of a benign breast cyst in 1973. On physical examination she appeared somewhat anxious and com- plained of chest pain. Eyes, ears, nose, and throat were normal. The neck was supple without palpable masses or venous distention. The chest was symmetrical and the expansion equal. Examination of the heart disclosed a loud, midsystolic click and high pitched late systolic murmur at the left sternal border and cardiac apex. The rhythm was of sinus origin with occasional episodes of flutter and sinus bradycardia with a rate of 33 beats to the minute. The blood pressure was 120/74 mm. Hg. The lungs were Vol. 54, No. 9, October 1978 892 M.L. GELFAND AND H. KLOTH PHONO ~i ..r-.-..,,_,,Cs:At, 4L.__,.._-.;\_. _iI*Fw_ Fig. 3: Prolapse of the posterior leaflet of the mitral valve clear to percussion and auscultation, and the rest of the physical examina- tion was within normal limits. Routine laboratory data were normal, except for a mild hypochromic anemia, leukopenia, and thrombopenia attributed to quinidine therapy. A phonocardiogram demonstrated a definite systolic click, and an echocardiogram revealed a prolapse of the posterior leaflet of the mnitral valve (Figures 5 and 6). While being monitored in the coronary care unit, she had multiple episodes of atrial flutter and periods of sinus arrest (Figure 7). Quinidine was discontinued and she was given propranolol 10 mg. four times a day. With this regime she developed more periods of Bull. N.Y. Acad. Med. 893 BRADYARRHYTHMIABRDARYHI I,i, 1VF77TtT 4 Fig. 4: A 24-hour Holter tape electrocardiogram showing a bradycardia with occasional ectopic ventricular contractions sinus arrest and marked bradycardia. Because of this, a permanent trans- venous pacemaker was implanted, and she was again given propranolol and digoxin with satisfactory control of all rhythm disturbances. She has remained free from chest pain and palpitations and has no bradycardia. DIscuSSION Mitral valve prolapse was initially thought to be a relatively rare, benign extracardiac phenomenon, but recently has emerged as an extremely com- mon disorder, not always benign.12 Its cause is uncertain and it is man- ifested by posterior displacement of one or both mitral leaflets across the atrioventricular groove into the left atrium during systole. The actual prevalence of this syndrome is at present unknown, but some reports indicate that it is a common echocardiographic finding in presumably healthy individuals. Markiewicz et al. in 19769 reported an incidence of 17% in 100 presumably healthy young women. Procacci et al.10 demon- strated mitral valve prolapse in 6.3% of 1,169 young, healthy women. Other observers have found this entity in young, asymptomatic subjects in appreciable numbers but less frequently in men.12 The clinical spectrum varies from a nonejection systolic click with or without a late systolic murmur and no mitral insufficiency to severe mitral incompetence with a holosystolic murmur and no click. The click, usually midsystolic, may be heard loudest at the cardiac apex, but can also be detected at the left sternal border or midprecordium. At times the click may be multiple, and at other times disappear entirely because the entity is Vol. 54, No. 9, October 1978 894 89 M.L.M..GLADADHGELFAND AND H. KLOTHLT \.'~~~~~~~~~~~7 I' 1L PARTS MM 181-112-082 MADE IN U.S.A.',e Fig. 5: Phonocardiogram showing a systolic click a dynamic one. The murmur is usually heard in late systole when present, and begins after a silent interval following the first sound. It may be introduced by a loud click and it often has a musical or "whooping" character. Various body positions and maneuvers augment the mur- mur.12"19 Passive leg raising increases venous return and increases the left ventricular diameter, delaying the murmur and click until later in systole. Standing decreases venous return and causes the murmur to lengthen and become louder. Squatting causes a simultaneous increase in venous return which increases ventricular size, delaying and shortening the murmur. The timing of the click and murmur is influenced by other specific manipula- tions, i.e., the Valsalva maneuver, exercise, and amylnitrate inhalation which also diminishes ventricular size, causing an early appearance of the murmur and click, whereas vasopressors delay the onset of both.18 The major clinical problems of the mitral valve prolapse syndrome consist of chest pain, palpitations, dyspnea and weakness, and ar- rhythmias. Chest pain is very common and usually atypical, unrelated to effort, stress, cold, food, or nitroglycerin. It also differs from true angina in that it lasts longer and does not radiate in the usual manner. Palpitations disturb the patient, often from a tachyarrhythmia, but more often they are unexplainable. Dyspnea and weakness may be manifestations of cryp- togenic abnormalities of left ventricular function or purely related to some as yet unrecognized neuropsychiatric disorder.7"7 Many patients with the systolic click-late systolic murmur syndrome have minor bony abnor- malities similar to those found in Marfan's syndrome, such as pes ex- cavatum, straight spine, and scoliosis.14,15 Bull. N.Y. Acad. Med. BRADYARRHYTHMIA 8959 -~~~~~~RDARYHI A,. (~ Fig. 6: Echocardiogram demonstrating prolapse of the posterior leaflet of the mitral valve About 25% of patients with this syndrome are entirely asymptomatic and the diagnosis rests on echocardiography which has contributed consid- erably to understanding the entity of mitral valve prolapse.